October, 10 2019

Hemoglobin Desaturation and RBC Adhesion: Potential Therapeutic Targets in Sickle Cell Disease

Locations

1 United States site

Age

> 18 Years

Genotypes

SS

About the study

As part of routine care for SCD, some people are found to have low oxygen levels (≤ 88%)
while sleeping, at rest, or with exercise. Testing is done with a small portable device
positioned on the finger that measures oxygen levels during sleep, at rest, or following
exercise. The investigators start oxygen treatment for people with low levels of oxygen. As a
part of this study, the investigators will find out if any changes in cell “stickiness” occur
with low oxygen levels (at rest, at night, or with exertion) and if cells become “less
sticky” with oxygen treatment. Study subjects will be seen before testing and 2 months after
testing. In some cases (people with low oxygen levels during testing), study subjects will
have been prescribed oxygen, and the investigators will test the effects of that treatment on
the stickiness of red cells.

Study Type

Observational

Age

> 18 Years

Genotype

SS

Gender

All

Inclusion Criteria

1. Male or Female > 18 year of age at the time of consent.

2. Documentation of Sickle Cell Disease, phenotypically HbSS (including S-Beta 0
thalassemia)

3. Written informed consent (and assent when applicable) obtained from subject or
subject’s legal representative and ability for subject to comply with the requirements
of the study.

4. English speaking patient

Exclusion Criteria

1. Ongoing Oxygen therapy.

2. active pregnancy, due to complex pathophysiology during that interval.

3. Presence of a condition or abnormality that in the opinion of the Investigator would
compromise the safety of the patient or the quality of the data. –

Outcome measures

  • Evaluation for exertional hypoxia
  • Evaluation for resting hypoxia
  • Evaluation of hypoxia and its effect on CBC
  • Evaluation of hypoxia and its effect on echocardiogram results
  • Evaluation of hypoxia and its effect on LDH
  • Evaluation of hypoxia and its effect on reticulocyte count
  • Evaluation of hypoxia and its effect on serum chemistry
  • Evaluation of patient's incidence of hypoxia-related nocturnal symptoms
  • Evaluation of patient's incidence of hypoxia-related symptoms
  • Examination of amount of baseline RBC adhesion and HEA in vitro in adults with HbSS
  • Examination of baseline FACS results in adults with HbSS
  • Examination of serial changes in amount of S-RBC adhesion at baseline and with hypoxia in vitro, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen
  • Examination of serial changes in amount of S-RBC adhesion at baseline and with hypoxia in vitro, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen.
  • Examination of serial changes in amount of WBC activation at baseline and with hypoxia in vitro, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen
  • Examination of serial changes in CBC at baseline and with hypoxia in vitro, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen.
  • Examination of serial changes in Hb saturation at baseline and with hypoxia in vitro, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen
  • Examination of serial changes in incidence of hypoxia-related symptoms at baseline and with hypoxia, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen.
  • Examination of serial changes in incidence of nocturnal symptoms at baseline and with hypoxia, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen.
  • Examination of serial changes in LDH at baseline and with hypoxia in vitro, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen.
  • Examination of serial changes in reticulocyte count at baseline and with hypoxia in vitro, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen.
  • Examination of serial changes in serum chemistry at baseline and with hypoxia in vitro, in adults with HbSS and resting or exertional hypoxia or NHD, before and after therapeutic intervention with oxygen.

Location

  • University Hospitals Cleveland Medical Center, Cleveland, Ohio, United States, 44106 [Recruiting]

Contact Research Team

  • Jane Little, MD
    216-844-1652

More info

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